The Family Adaptation study, ancillary to the Single Ventricle Reconstruction Trial, examined the prevalence of anxiety and its associations with stress, psychosocial factors, and quality of life measures in parents of infants who underwent the Norwood procedure. Two hundred and fifteen parents (143 mothers and 72 fathers) of 146 infants completed state anxiety (State Anxiety Inventory), stress, psychosocial, and quality of life measures post-Norwood, post-Stage II, and at a final visit (median child age: 14 months). A substantial proportion of parents reported severe anxiety symptoms following the Norwood surgery, with 61% of mothers and 43% of fathers affected, decreasing over time to 46% and 33% by the final visit, respectively. Mothers' average STAI-S scores were significantly higher than fathers' post-Norwood (47.7 ± 13.2 versus 43.5 ± 11.8, p = 0.03), declining to 42.1 ± 13.0 versus 39.0 ± 9.6 (p = 0.14) at the final visit. Stress related to parenting a child with a serious illness was a stronger and more consistent predictor of mothers' anxiety over time (highest R2 = 0.49 for emotional distress), whereas insufficient coping and fewer protective factors were greater and more consistent predictors for fathers (highest R2 = 0.40 for mastery and health). Quality of life was a consistent predictor of state anxiety for both mothers and fathers. Anxiety is elevated in parents of infants who underwent the Norwood procedure and is influenced by a complex interplay of stress, psychosocial factors, and quality of life. Addressing these factors is crucial for improving parents' mental health, which in turn promotes the well-being of the entire family.

Right ventricular dysfunction is a major determinant of long-term outcomes after tetralogy of Fallot repair, particularly in the presence of chronic pulmonary regurgitation. Strain analysis may detect early right ventricular dysfunction more sensitively than conventional echocardiographic parameters. This retrospective single-centre study included 63 patients after tetralogy of Fallot repair who underwent postoperative echocardiographic evaluation during mid-term follow-up. Right ventricular function was assessed using tricuspid annular plane systolic excursion, right ventricular systolic velocity, fractional area change, and right ventricular free-wall longitudinal strain. Pulmonary regurgitation severity was evaluated by colour Doppler, with pulmonary regurgitation jet width expressed as a ratio relative to the pulmonary annulus diameter. Associations between pulmonary regurgitation severity and right ventricular function were analysed using correlation and multivariable linear regression. Conventional right ventricular systolic parameters were largely preserved, whereas right ventricular free-wall longitudinal strain worsened with increasing pulmonary regurgitation severity (p = 0.036). Pulmonary regurgitation jet width emerged as the sole independently associated determinant of impaired right ventricular strain (β = 0.132, p = 0.038). QRS duration was not independently associated. Pulmonary regurgitation jet width showed moderate discriminatory ability for impaired right ventricular strain (area under the curve = 0.67). Right ventricular free-wall longitudinal strain is a sensitive marker of pulmonary regurgitation-related right ventricular dysfunction after tetralogy of Fallot repair. Pulmonary regurgitation jet width, expressed relative to the pulmonary annulus, appears to be the dominant determinant of right ventricular mechanical impairment and may complement conventional assessment during follow-up.

Dilated cardiomyopathy is a rare but severe myocardial disease in the paediatric population, often leading to heart failure, heart transplantation, or sudden cardiac death. Genetic factors are a major contributor to childhood dilated cardiomyopathy. Recently, biallelic variants in the PPP1R13L gene have been implicated in a novel syndromic form of early-onset dilated cardiomyopathy, characterised by cardiac dysfunction alongside variable ectodermal features. We report a 4-year-old boy who presented with decompensated heart failure and echocardiographic findings consistent with dilated cardiomyopathy. Syndromic features included sparse, dry hair, high anterior hairline, broad nasal bridge, and pointed teeth. Genetic analysis revealed a novel homozygous frameshift variant in the PPP1R13L gene (c.2368_2375dup; p. Pro793Glyfs*32), classified as pathogenic. The clinical course was complicated by recurrent ventricular arrhythmias and ultimately sudden cardiac death. PPP1R13L-related cardiomyopathy should be considered in children with early-onset dilated cardiomyopathy and syndromic features. Early diagnosis is critical for clinical management, arrhythmia surveillance, and appropriate family counselling.

Although acute type A aortic dissection (ATAAD) is a recognised complication of Turner syndrome, population-level data describing its prevalence and outcomes remain limited. We queried the National Inpatient Sample (2002-2020) and generated three groups: TS + ATAAD+, TS + ATAAD-, and TS-ATAAD+ in our retrospective observational cohort study. Primary outcomes were ATAAD hospital admission rate and in-hospital mortality; secondary outcomes included length of stay. Sampling weights were applied to all analyses. A total of 51,426 Turner syndrome hospitalisations were identified. Median ages were 40 years (interquartile range 31-50) for TS + ATAAD+ and 32 years (interquartile range 9-49) for TS + ATAAD- (p < 0.001). Overall, 0.27% of Turner syndrome hospitalisations presented with ATAAD. Compared with TS + ATAAD-, those with ATAAD had higher mortality (13.1% vs 2.1%, p < 0.001) and longer length of stay (median 8 vs 3 days, p < 0.05). Rates of bicuspid aortic valve (34.3% vs 3.5%, p < 0.001), hypertension (46.0% vs 25.7%, p < 0.05), and complete heart block (14.6% vs 0.8%, p < 0.001) were also higher. Multivariable analysis showed bicuspid aortic valve (adjusted odds ratio 24.31, 95% CI 8.8-67.3) and age (adjusted odds ratio 1.02, 95% CI 1.01-1.04) predicted ATAAD. Compared with TS-ATAAD+ patients, those withTS + ATAAD+ were younger (40 vs 68 years, p < 0.001), while mortality, length of stay, hypertension, and bleeding rates were similar. ATAAD is highly lethal in patients with and without Turner syndrome. In Turner syndrome, ATAAD is associated with higher mortality, longer hospitalisation, and higher rates of bicuspid valve disease and hypertension.

A previously asymptomatic 7-year-old boy with Duchenne muscular dystrophy unexpectedly developed sudden cardiac arrest with no preceding illness. An automated external defibrillator confirmed the presence of ventricular fibrillation. Cardiac MRI showed prominent myocardial fibrosis with no evidence of acute inflammation. Whole exome sequence revealed no associated pathological variance for lethal ventricular arrhythmias. Life-threatening ventricular fibrillation can occur in young patients with Duchenne muscular dystrophy.

Cantrell's pentalogy is a rare congenital disorder with high mortality. This report presents an infant with type 1 Cantrell's pentalogy and complex CHD, managed through staged surgeries and hybrid interventions. Genetic studies ruled out hereditary causes. The case highlights the importance of multidisciplinary approaches and further documentation to improve management and outcomes.

Studies assessing the effect of preoperative iron supplementation in paediatric cardiac surgery are limited and yield conflicting data. The study aimed to evaluate the effect of preoperative oral iron supplementation on allogeneic blood transfusion after cardiac surgery for acyanotic CHD. This was a prospective, open-label, outcome assessor-blinded, randomised clinical trial performed in a large tertiary care centre in India. Children (haemoglobin <13 gm/dl) with acyanotic CHD were recruited for the study. Children in the intervention arm received colloidal iron (3 mg/kg), folic acid, and cyanocobalamin according to body weight for at least 7 days before cardiac surgery. The primary outcome was the amount of allogeneic blood transfusion, while secondary outcome measures were the duration of mechanical ventilation and ICU stay. A total of eighty-six children (43 in each arm) completed the trials. A total of 43 children received iron supplementation for a minimum of 1 month. The haemoglobin level improved by 1.3 gm/dl immediately before the cardiac surgery in the intervention arm. The allogeneic blood transfusions (ml/kg) median (IQR) were significantly lower in the intervention group (5 (0–8) vs 10 (8–12); p < 0.01). Furthermore, total cumulative allogeneic blood transfusion was also significantly lower in the intervention arm (60 ml; (0–100) vs 100 ml; (70–140); p < 0.001). The duration of mechanical ventilation, ICU stay, and hospital stay was significantly lower in the intervention arm. Preoperative oral iron supplementation significantly reduces the need for allogeneic blood transfusion in children undergoing cardiac surgery for acyanotic heart disease.

Infants with single ventricle CHD commonly experience gross motor delays due to physiological and environmental factors, including increased risk for white matter injury, reduced aerobic capacity, restrictive post-operative protocols, and limited movement opportunities. These delays persist in adolescence, affecting physical and social development. This study examines a quality improvement initiative within the National Pediatric Cardiology Quality Improvement Collaborative to enhance gross motor development. Fifteen centres participated. Gross motor skills were assessed using the Ages and Stages Questionnaires, Third Edition, at 6 and 12 months. A key driver diagram, Plan-Do-Study-Act cycles, baseline data, and ongoing process measures were collected. Interventions were implemented, including education, therapy support, and mobilisation protocols. Control charts were used to evaluate the data. Scores from the Ages and Stages Questionnaires indicated delays in gross motor skills at baseline, with improvement over time. Participating centres showed a centerline shift from 41% to 89% of infants achieving on-target or improved motor scores, compared with 54% to 68% of infants at non-participating centres. Establishment of customised infant developmental plans increased from 62% to 74% for participating centres and from 53% to 61% for non-participating centres. Interventions included establishing processes for consistent screening, developmental plan administration, review of prone positioning, access to therapies, and early intervention referrals. This targeted quality improvement project increased the use of inpatient practices to support gross motor development for infants with single ventricle CHD. Collaborative, interdisciplinary efforts remain critical for addressing neurodevelopmental challenges in this high-risk population.

Chylothorax and chylopericardium are rare in children and are typically associated with cardiothoracic surgery, congenital lymphatic abnormalities, or malignancy. Traumatic chylous effusions are particularly uncommon. We report an 11-year-old boy who developed extensive cervical and upper chest swelling following a minor firecracker blast injury. Subsequent imaging revealed large chylous pleural and pericardial effusions. Persistent high-output chyle loss despite exhaustive conservative therapy necessitated thoracic duct embolisation, which successfully resolved the effusions. This case highlights the importance of considering lymphatic injuries following seemingly trivial trauma and demonstrates the utility of percutaneous thoracic duct embolisation in paediatric lymphatic leaks.

The KONAR-MF™ occluder, with its flexible medium-profile design, has broadened the feasibility of transcatheter closure of muscular ventricular septal defects, particularly in infants. To assess feasibility, safety, techniques, and outcomes of muscular ventricular septal defect closure using the KONAR-MF™ occluder in a multicentre paediatric cohort. A retrospective review was conducted at three tertiary paediatric cardiac centres (2018-2024). Patient demographics, ventricular septal defect characteristics, procedural approaches, and follow-up outcomes were analysed. Device implantation was performed via retrograde, antegrade, transseptal, or hybrid approaches under fluoroscopic and echocardiographic guidance. Fifty patients (54 devices) were included (median age: 48 months [interquartile range 12-96]; weight: 12 kg [interquartile range 7.5-23]), including 14 infants (9 < 7 kg). Indications were failure to thrive (46%), heart failure (28%), recurrent infections (12%), and postoperative residual ventricular septal defect (14%). The mean ventricular septal defect size was 5.8 ± 2 mm. Median fluoroscopy time was 18 minutes (range: 3-71). Residual shunts were present in 18% immediately, reducing to 9% at one week and resolving by three months. Mild, transient tricuspid regurgitation occurred in 14%. Over a median 9-month follow-up (range 1-60), no cases of heart block or haemolysis occurred. One embolisation required surgical retrieval. Pulmonary artery pressure decreased significantly (37 ± 13.4 to 19 ± 3.8 mmHg, p < 0.001). Transcatheter closure of muscular ventricular septal defects with the KONAR-MF™ occluder is safe, effective, and versatile across paediatric age groups, including infants and postoperative cases. High success rates, minimal complications, and favourable short- to mid-term outcomes support its use in routine practice.